RECURRENT PSEUDO-OBSTRUCTION OF THE LARGE BOWEL ASSOCIATED WITH SANJAD-SAKATI SYNDROME: A CASE REPORT

Abstract

Sanjad-Sakati Syndrome, also referred to as Hypoparathyroidism-Retardation-Dysmorphism Syndrome or Middle East Syndrome, was first reported in Saudi Arabia in 1988 and later in a definitive report by (Sanjad et al.) in 1991. It is a rare autosomal recessive condition reported almost exclusively in the Arab population. The condition is characterized by an extensive array of symptoms, including congenital hypoparathyroidism, severe growth and developmental retardation both intrauterine and post-natal, low Intelligence, seizures, and distinctive facial characteristics. We present an incidence of this disease characterized by repeated pseudo-obstruction. Electrolyte and metabolic disturbances, which are the cause of pseudo-obstruction of the intestine in this child, must be evaluated in order to avoid unnecessary surgical intervention, which may prove to be life threatening.